Lymphoedema is an accumulation of lymph in the interstitial spaces caused by a defect in the lymphatic drainage system. Reduced lymph drainage can result from either congenital or acquired anomalies of the lymphatic system. Although lymphoedema usually affects one or more extremities, is can also manifest itself in the organs. Regardless of the pathogenesis, it is often a chronic, intractable condition that becomes a long-term physical and psychological problem for patients and a complex therapeutic challenge for doctors.
In many cases of advanced lymphoedema, we find a typical medical history with a characteristic clinical presentation that enables almost certain diagnosis using basic diagnostics comprising a preliminary case history, inspection and palpation, in which a positive Stemmer’s sign is evidence of the disease. Robert Stemmer showed that the dorsal skin fold at the base of the second toe in healthy patients can be lifted from the bone while this is not possible in patients with distal lymphoedema. A positive Stemmer’s sign is a definite indication of lymphoedema, however if Stemmer’s sign is negative, the patient may still have a proximal lymphoedema. In this case, further examination is necessary. Diagnosis is more difficult in the less severe stages of oedema. Available tests include isotope lymphoscintigraphy, direct and indirect lymphography, fluorescence micro lymphography, magnetic resonance tomography, computed tomography and ultrasound.
We differentiate between three forms of primary lymphoedema:
-Congenital lymphoedema occurs at birth or within the first two years of life.
-Lymphoedema praecox is the most common form of primary lymphoedema and occurs around puberty or in young adults up to age 35.
-Lymphoedema tarda occurs in patients aged 35 or older and requires extreme care in diagnosis to ensure that a secondary lymphoedema caused by a tumour-related obstruction is not overlooked.
Secondary lymphoedema develops as a result of damage to the lymphatic pathways and vessels or lymph nodes or from external compression, surgery or various disease processes.
Chronic lymphoedema often entails complications. In the case of a lymphoedema that has persisted for decades, a variety of neoplastic complications can also arise.
Lymphoedema is a chronic disease that requires lifelong treatment. That does not mean that lymphoedema is incurable. It can only be managed. A great number of treatment options are now available that have efficiently reduced oedemas and prevented fluid accumulation. On the other hand, when treatment is discontinued swelling inevitably increases and the patient’s condition worsens considerably following recurring infection as a result of the massive oedema. In addition, patients may experience severely restricted mobility of their extremities accompanied by physiological effects on their everyday life and even life-threatening infections such as erysipelas or malignant complications.
Complex physical decongestion (CPD) therapy
Complex physical therapy is done in 2 phases:
In phase 1 of decongestion, meticulous hygiene is necessary and particular care must be given to digital mycosis, small wounds and cracks in the skin. In addition, phase 1 includes whole-body manual lymph drainage (MLD) up to 5 times per week in keeping with the principles described by Winiwarter, Vodder und Földi followed by compression bandaging and physiotherapy movement exercises and terrain training.
In phase 2 of decongestion, the frequency of manual lymph drainage can be reduced to 2 times per week in an outpatient setting. The patient is then provided with a compression sleeve that includes the shoulder or a compression pantyhose. Sometimes, outpatient manual lymph drainage may be followed by compression bandaging to further promote decongestion.
The maintenance phase seeks to maintain the results achieved through decongestion.
Low-stretch bandages are used to reduce oedema during the decongestion phase. Low-stretch bandages provide high working pressure and low resting pressure to ensure drainage.
Compression garments make treatment easier for the patient during the maintenance phase since low-stretch bandages are difficult to apply and tend to slip during daily use. Manufacturers have recently made considerable improvements in flat-knit products. Because compression garments lose their compression capacity after 3 to 6 months, they must be replaced accordingly. Lower-grade compression garments provide compression of up to 40 mm Hg while higher-compression garments provide 59 mm Hg or more.
Pharmacological therapy and diet
Coumarin has proven to slowly reduce various forms of lymphoedema in several studies conducted on both humans and animals. In some cases, the administration of selenium is being considered as it is thought to have a positive effect in reducing the frequency of recurrent erysipelas. There is no specific diet for lymphoedema. However, patients should reduce consumption of items that increase capillary pressure such as coffee, red wine and sparkling wine. Cutting back on proteins does not improve lymphoedema but rather adds to the problem by causing hypoproteinaemic oedema.
Surgery is done when other customary medical therapies have failed. There are two main surgical interventions, 1) reduction plastic surgery and 2) microsurgical lympholymphatic, lymphovenous or lymph node-venous anastomosis.
using infrared light
Lipoedema is a disorder of the distribution of fat within the body. It occurs most frequently on the upper thigh and lower leg and involves orthostatic oedema in the latter half of the day as well as pain that is difficult to localize. If these three elements are absent, the disorder at hand is lipohypertrophy. The swelling characteristically ends at the ankle level. The arms are more rarely affected. The first signs of lipoedema generally appear toward the end of puberty, but the illness can start at any age. Sometimes lipoedema occurs following trauma. For example, incidence of lipoedema in female patients who have suffered extensive burns has been observed.
Lipoedema can be hereditary. Ultimately, the exact cause of lipoedema is unclear. The disease occurs almost exclusively in women, which suggests that hormonal factors play a considerable role combined with a hereditary tendency. Free radicals can also trigger lipoedema after accidents and if similar triggering factors come into play.
Subcutaneous fat in the legs is always symmetrical. Typical localizations include increased fatty tissue on the buttocks and hips as well as fatty bulges in the medial proximal (upper) thigh and the medial distal thigh (just above the knee). In later years, the fat can reach to the ankles and can alter the shape of the leg so that it has the appearance of a column. If the ankle area, feet and toes are free of swelling and Stemmer’s sign is negative, the patient does not have lymphoedema. In some cases, the arms are also affected. If untreated, an accompanying lymphoedema can occur with a latency period of 15 to 18 years.
The condition is categorized in different stages according to the degree of severity.
Stage 1: Cellulite, finely dimpled skin surface
Stage 2: “Mattress skin,” coarsely dimpled skin surface with large indentations
Stage 3: Large, deforming folds of fat
Diagnosis is usually made by taking a medical history and doing an inspection and palpation. Lipoedema can be identified solely on the basis of its appearance and feel. The typical distribution patterns of the subcutaneous fat on the hips, thighs, knees and ankle region and the lack of swelling in the feet indicate lipoedema. Orthostatic oedema occurs in the latter half of the day. Painfulness to the touch or to pressure applied to the front edge of the shin also indicates lipoedema.
The arterial reserve is limited because even the smallest arteries cannot sufficiently circulate blood in the subcutaneous fat in this tough, hard fat tissue.
Sonography of the lower extremities reveals a symmetrical distribution of the subcutaneous fat tissue, with a “snow flurry”-like change in the subcutaneous fat tissue.
Computed tomography (CT):
Shows a marked thickening of the subcutaneous fat tissue, as does magnetic resonance tomography (MRT)
Shows typical changes in the lymph capillaries with aneurysm-like bulges
A scintigraphy in which the absorption of particles by the lymph nodes is measured in accordance with a predefined sequence of movement. This test method can be used to determine the existence of an accompanying lymphoedema.
The treatment regime used will depend on the stage of the lipoedema:
Few patients come for treatment in stage 1, in which the cosmetic aspect dominates. Any existing overweight is to be treated and athletic activities are to be carried out in combination with compression of the affected parts.
In stage 2, the focus is more on the increase in the circumference of the affected parts and deformations of contours and of the skin surface. The oedemas increase and pain can be considerable. When the complete range of symptoms exists, with fat distribution, orthostatic oedemas and pain, all therapy options should be used.
In stage 3, the use of psychotherapy in addition to physical therapy should be considered.
- Diet (to reduce the excess weight that accompanies the condition)
- Aerobic exercise using compression
- Complex physical decongestion (CPD) therapy / manual lymph drainage (MLD)
- Intermittent equipment-based compression therapies
- Compression garments
- Corrective surgery (lymphological liposuction)
Compression garments – classes and types of compression
Compression garments can be circular-knit or flat-knit. Circular-knit garments are knit without a seam and with the same number of stitches throughout the length of the garment, much like normal stockings. The different circumferences along the leg are achieved by pre-stretching the elastic thread to different degrees so that the weave is thinner in those areas. Flat-knit garments are produced according to a row-by-row knit pattern. The different circumferences along the leg are achieved by varying the number of stitches per row. This knitting method makes it possible to produce otherwise unthinkable forms and size variations that fit perfectly. The garments fit perfectly and provide the exact compression that is needed, where it is needed. Flat-knit garments are not as easily overstretched as circular-knit garments.
Dr. F.-J. Schingale